Gheita T A, Abd El Latif E M
Rheumatology and Clinical Immunology, Faculty of Medicine, Cairo University, Cairo, Egypt.
Ophthalmology Department, Faculty of Medicine, Alexandria University, Alexandria, Egypt.
Z Rheumatol. 2019 Apr;78(3):281-286. doi: 10.1007/s00393-018-0495-5.
The aim of this study was to assess disease characteristics, autoantibodies, and disease activity in granulomatosis with polyangiitis (GPA) patients with ocular manifestations.
The cohort included 46 GPA patients visiting the ophthalmology clinic. Ocular manifestations were recorded, clinical and slit lamp examinations were performed. The Birmingham Vasculitis Activity Score (BVAS) was recorded. Laboratory investigations and the antineutrophil cytoplasmic antibody (ANCA) assay were performed.
Median age of the 22 male and 24 female patients was 44.5 (32-63) years, median disease duration 6.5 (1-16) years. Ocular manifestations were present in all patients: 12 (26.1%) had proptosis; 40 (87%) had scleritis/episcleritis, with perforation in 3 (6.5%); 33 (71.7%) had keratoconjunctivitis (KC), with acute infiltrative stromal keratitis in 11, peripheral ulcerative keratitis in 15, and sclerosing keratitis in 11 patients. Uveitis was present in 11 (23.9%) and retinal changes including vasculitis, exudates, and hemorrhage were present in 7 (15.2%). Blurred vision was present in 43 (93.5%) patients and 2 (4.3%) had vision loss. Glaucoma was present in 4 (8.7%) and hypotony in 2 (4.3%) patients. Involvement was bilateral in 32 (69.6%) patients. Rheumatoid factor (RF) was positive in 56.5% and significantly associated with uveitis (p = 0.04), while antinuclear antibody (ANA) was positive in 45.7% and significantly associated with KC (p = 0.04). BVAS tended to be higher in patients with uveitis (p = 0.49).
Ocular involvement must be considered in all GPA patients and referral to an experienced ophthalmologist is mandatory for proper management and improved outcome of such a rare systemic disease. ANA and RF positivity may raise suspicion for KC or uveitis, respectively. There was a remarkable association between uveitis and disease activity.
本研究旨在评估有眼部表现的肉芽肿性多血管炎（GPA）患者的疾病特征、自身抗体及疾病活动度。
该队列包括46例到眼科门诊就诊的GPA患者。记录眼部表现，进行临床及裂隙灯检查。记录伯明翰血管炎活动评分（BVAS）。进行实验室检查及抗中性粒细胞胞浆抗体（ANCA）检测。
22例男性和24例女性患者的中位年龄为44.5（32 - 63）岁，中位病程为6.5（1 - 16）年。所有患者均有眼部表现：12例（26.1%）有眼球突出；40例（87%）有巩膜炎/表层巩膜炎，其中3例（6.5%）出现穿孔；33例（71.7%）有角结膜炎（KC），其中11例为急性浸润性基质性角膜炎，15例为周边溃疡性角膜炎，11例为硬化性角膜炎。11例（23.9%）有葡萄膜炎，7例（15.2%）有包括血管炎、渗出物及出血在内的视网膜改变。43例（93.5%）患者有视力模糊，2例（4.3%）有视力丧失。4例（8.7%）有青光眼，2例（4.3%）有低眼压。32例（69.6%）患者为双侧受累。类风湿因子（RF）阳性率为56.5%，与葡萄膜炎显著相关（p = 0.04），而抗核抗体（ANA）阳性率为45.7%，与KC显著相关（p = 0.04）。葡萄膜炎患者的BVAS往往更高（p = 0.49）。
所有GPA患者均须考虑眼部受累情况，对于这种罕见的全身性疾病，必须转诊至经验丰富的眼科医生处进行恰当管理并改善预后。ANA和RF阳性可能分别提示KC或葡萄膜炎。葡萄膜炎与疾病活动度之间存在显著关联。
